Patient Services
What Is ALS?
Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease, first identified in 1869 by the noted French neurologist Jean-Martin Charcot. Although the cause of ALS is not completely understood, the 1990's have brought a wealth of new scientific understanding about the physiology of this disease.
Lou Gehrig, with whom ALS is most commonly associated, first brought national and international attention to the disease back in 1939 when he abruptly retired from baseball after being diagnosed with ALS. To learn more about Lou Gehrig, visit www.lougehrig.com.
However, ALS is not just Lou Gehrig's disease and it knows no boundaries. The disease has cut short the lives of such notable and courageous individuals as Hall of Fame pitcher Jim "Catfish" Hunter, Senator Jacob Javits, actors Michael Zaslow and David Niven, creator of Sesame Street Jon Stone, boxing champion Ezzard Charles, NBA Hall of Fame basketball player George Yardley, pro football player Glenn Montgomery, golfer Jeff Julian, golf caddie Bruce Edwards, British soccer player Jimmy Johnstone, musician Lead Belly (Huddie Ledbetter), photographer Eddie Adams, entertainer Dennis Day, jazz musician Charles Mingus, composer Dimitri Shostakovich and U.S. Army General Maxwell Taylor.
For more information, please visit About ALS.
How can I find a doctor knowledgeable and experienced in taking care of people with ALS?
Selecting a physician to manage your ALS care will often be influenced by what is covered by your health insurance plan. If necessary, contact your insurance company to request approval to receive care at an ALS specialty clinic or center.
Finding a doctor who is a "match" for you and your family is important. Individual styles and approaches to care will vary - despite equal medical skill and competence. Do not hesitate to change physicians, if necessary, to find someone with whom you can communicate and who respects health care decisions you will make in dealing with ALS. Openly discuss your needs and thoughts with your physician and members of the health care team and be sure to ask questions.
- First, refer to the list of certified ALS Association CentersSM found in this web site. These sites provide specialized ALS care, services and information to assist people with ALS and their families in living as fully as possible. Simply call the number listed or contact the clinic by mail or fax to make an appointment.
The ALS Association has developed a program to certify and recognize ALS clinics that meet the criteria of the ALS Association CenterSM program. The criteria were established to define, establish and support a national standard of care in the clinical management of ALS.
Criteria:
Because ALS involves physical and psychological functioning in many different ways, a multidisciplinary team approach to care provides patients and families the most comprehensive, well-planned approach to care. By building a team of ALS clinical experts in physical therapy, speech, occupational therapy, nursing, psychology, social work, nutrition and other fields, the ALS neurologist can coordinate a plan of care that brings all health care professionals together to see the patient and family at one visit.
ALS Association Certified CentersSM conduct basic, clinical or drug trial research.
The ALS Association Center medical director is a well-recognized expert in ALS.
A full range of neurological diagnostic equipment and other necessary medical services
Staff at the ALS Association CenterSM take care of a number of people with ALS and have developed a specific clinic service for ALS patients
- Second, refer to the list of ALS Clinics that work closely with one of The ALS Association's Chapters. This list can be located on The ALS Association's web site.
Local ALS Association Chapters have reached out to some of the best ALS physicians and clinics in their community to work closely with the clinic staff in meeting the needs of people with ALS and their families. These clinics provide excellent clinical care and many are modeled after the criteria of the ALS Association CenterSM program.
- Third, The ALS Association web site includes a list of physicians throughout the country who take care of people with ALS. This list is complied from names provided by local ALS Association Chapters and Free Standing Support Groups.
- Fourth, The World Federation of Neurology maintains an international list of ALS research and clinical centers on its web site www.wfnals.org
It is not uncommon that a person with ALS will live in a geographic area without any expert ALS clinicians. In these situations, the medical directors of the ALS Centers and clinics work closely with the patient's local medical doctor, the patient and family to coordinate care locally. This approach brings the best of both worlds. The patient and family can receive most of the care and services they need in their local community comforted by the knowledge that they and their local doctor are being guided by the nurse, doctor, therapists and others from the large specialty ALS center. Generally, two or three times a year the patient will be seen at the referral specialty clinic for re-evaluation and any necessary tests or therapies.
Why is it important to get a second opinion?
Your doctor has told you that you have ALS. Getting a second opinion on the diagnosis of a serious and ultimately life-threatening disease like ALS can be critical. Since there is no definitive test for ALS and the symptoms can mimic many other medical conditions, making the diagnosis of ALS can be difficult. Often it is only after the symptoms have progressed and the patient shows conclusive signs and symptoms of the disease that a physician can reach a diagnosis of "definite" ALS. Some neurologists estimate that as many as 15% of the people diagnosed with ALS have been incorrectly diagnosed.
You think you may have the symptoms of ALS, but no one has given you this diagnosis. It is not uncommon to hear of people eventually diagnosed with ALS who had progressive muscle weakness, twitching or cramps for a year or more without knowing what was wrong. In some situations, patients undergo treatments including surgery and other serious and expensive medical interventions for misdiagnosed symptoms. If the symptoms of muscle weakness, twitching or cramps progress despite therapies, or your doctor can't reach a diagnosis, it may be time to get a second opinion. It's important, however, not to become unduly alarmed. ALS is an uncommon disease with only 2 cases diagnosed per 100,000 population each year. This translates into approximately 5,000 new cases of ALS in the U.S. each year.
As quoted from an article in the Los Angeles Times ("Need a Second Opinion?" by Rosie Mestel. Los Angeles Times. February 15, 1999, Home Edition: Health Section, Page S-1),
" ' Whenever you have doubts about your care, you should at least think about getting a second opinion,' says Peter Lee, director of consumer protection programs at the Center for Health Care Rights, a Los Angeles-based consumer advocacy group. 'Patients aren't shackled to their doctors. They need to be assertive. They need to be in control of deciding what their care should be.' "
How to go about getting a second opinion
First, let your current physician know you'd like a second opinion. Since this is becoming a common occurrence in the U.S. and most doctors truly have your health as their primary concern, you can likely expect your doctor to be open to this idea. However, it's normal to feel a certain amount of reluctance to bring up the question of a second opinion to your doctor. Most Americans have confidence in their health care provider and don't want to risk offending their physician or jeopardizing the relationship they have established.
One of the tips from the Los Angeles Times article suggests, " … say something like, 'You know, this is a complicated and important (issue) for me, and I think I'd like to talk to another physician about my (diagnosis) as well. Perhaps you have a recommendation?' That helps preserve the bond', says Peter Clarke, professor of preventative medicine at USC and co-author of "Surviving Modern Medicine."
Next, find a doctor for your second opinion. Since even some neurologists haven't taken care of a patient with ALS, is important to consider seeing a sub specialist - a neurologist specializing in neuromuscular diseases like ALS. In the approximately 60 ALS clinics across the country, there are physicians knowledgeable and experienced in making the diagnosis of ALS. Your current doctor may be able to recommend someone. Included on The ALS Association's web site are lists of certified ALS Association CentersSM and ALS clinics working with The ALS Association chapters. Other places to get information include your local ALS Association chapter or support group, The ALS Association's national toll-free information and referral service (800) 782-4747 or the many reliable medical Internet web sites. Other sources of information are local hospitals, state medical and neurological associations and the American Academy of Neurology. An advantage of consulting a doctor who treats a number of ALS patients is that he or she is likely to be informed about the latest treatments and current research and can offer a supportive, hopeful perspective.
Who pays for a second opinion?
Before making a final selection on whom to see for your second opinion, review your health insurance policy or contact your health insurance company to find out if the cost of a second opinion is covered under your policy. Also check to learn if there are any restrictions as to which physician can provide a second opinion. Often managed care companies will require that patients to get their second opinion from a doctor who participates in the same managed care plan. The policies on coverage for second opinions vary from state to state and company to company. Some states are considering legislation that would require health care plans to pay for a second doctor's opinion if the patient or primary doctor requests one. Second opinion physician consultations can cost $150 or more if patients have to pay for the visit themselves. In dealing with health insurance companies, remember that patients have the right to appeal insurance decisions. For more information about appeals in managed health care companies, contact the state Department of Corporations.
What to expect at your second opinion appointment
- Let the doctor's office know you are requesting a second opinion for an ALS diagnosis. Many doctors will schedule an appointment for ALS consultation more quickly than routine appointments.
- Often the consulting physician will be able to use the results of test you have already had thus saving money, time and the process of repeating multiple tests. Be sure your complete medical record, results of tests, and actual MRI, CT, or X-ray films and electromyography (EMG) recordings get to the doctor's office before your appointment - or bring them with you. By having the actual results rather than the report of the test, the consulting physician can provide his or her own interpretation of the test results.
- It's a good idea to have a relative or friend come with you for support and to take notes on, or tape record, what the doctor says and recommends. Realize that a second opinion appointment can be a stressful time and be sure to write out your questions in advance.
- Since you will likely be seeing an ALS expert, take advantage of the opportunity to ask questions about prognosis (what your future holds), research, treatment, availability of drug trials and, most importantly, how to manage ALS symptoms and maintain your physical function for as long as possible.
- Verify that the consultant will provide a full report to your current doctor and will be available to you for follow up questions. "'Education is empowerment,' says Cedars-Sinai's Beth Karlan, director of gynecologic oncology. 'It's a sound bite, but it's true. It's really important that you view yourself as a consumer of the most important thing in the world - your own life.'"
"Need a Second Opinion?" by Rosie Mestel. Los Angeles Times. February 15, 1999, Home Edition: Health Section, Page S-1.
"Preparing for Your Second-Opinion Visit." By Rosie Mestel. Los Angeles Times. February 15, 1999. Home Edition: Section Health, Page S-5.
How can I manage the problem of too much saliva?
You are not alone. Many people with ALS experience pooling of saliva in their mouths that can make people more likely to drool, choke or cough. It may seem as though your body is producing too much saliva, but it is not. Normally, our bodies produce large quantities of saliva each day to help maintain a healthy mouth and good digestion. For reasons that are not clearly understood, you may not be swallowing your saliva as automatically (without thinking) as you did prior to ALS. For some people, the difficult swallowing that can be a part of ALS makes it harder to keep the mouth clear. In addition, you may have more saliva when you are hungry, anxious or smell good food.
If your saliva is bothersome, there are several medications that can help dry your mouth. Ask your doctor or nurse about using one or more of the following: Robinul©, Elavil©, Benadryl©, Ditropan©, Atropine, Cogentin©, Artane© and Levsin©.
Some people get improvement from small amounts of meat tenderizer, as one of the ingredients tends to dry the mouth. Other people use suction machines and large, plastic suction tubes to keep their mouths clear. Portable suction machines are available for rental or purchase. For certain situations, using absorbent cloths or paper towels to clear the mouth can provide temporary control of excess saliva.
Living with ALS Manual No. 3 - "Managing Your Symptoms and Treatment"
Deborah Gelinas, M.D.
Forbes Norris ALS Clinic and Research Center
San Francisco, CA
Practice Parameter: The Care of the Patient with ALS
Report of the Quality Standards Subcommittee of the American Academy of Neurology
January 1999
Can creatine help people with ALS and what are the potential side effects?
As with any medication or dietary supplement, it is important to check with the physician managing your ALS care to discuss the pros and cons of adding any new drug or supplement. Creatine is a dietary supplement and is sold in most health food stores. A variety of companies produce creatine. Because it is not a drug, creatine does not fall under the control of the FDA for product claims or manufacturing purity. For a list of centers conducting clinical trials with creatine, go to The ALS Association's Drug Development Update on this web site.
"Recent studies in the transgenic animal models of ALS (model of familial ALS) provide evidence that creatine is effective in protecting degenerating motor neurons and delaying disease deterioration in these mice. Clinical trials are of the utmost importance to investigate if the effects seen in the mouse model are seen in humans with ALS. Another question is whether this exciting result found in a model of familial ALS is applicable to sporadic ALS that constitutes more than 90% of all ALS."
Hiroshi Mitsumoto, M.D.
Columbia University
New York, New York
Formerly of the Cleveland Clinic
"Drugs and nutritional supplements that can help muscular function are one approach to getting symptomatic improvement in ALS and other muscular problems. Athletes and body-builders have long used creatine.
Can it also be useful in people with muscle diseases? … The current studies in people without neuromuscular disease suggest some types of muscle performance may be improved by creatine supplementation. In people without neuromuscular disease, there has been concern with regard to safety due to potential (kidney) problems if adequate (fluid intake) is not maintained. The deaths of three wrestlers who were on creatine in the last few years has raised our concern about what are the best ways that this substance may be safely given. Some sports medicine experts and trainers are unsure about potential side effects and benefits of long term usage. …
Certainly, many ALS patients take a large number of nutritional supplements without any published evidence of benefit, usually without a physician's prescription or advice. Since many ALS patients have dysphagia (difficulty swallowing), and may tend to be mildly dehydrated, they should be cautioned about the potential danger of taking creatine if they cannot take in adequate fluids.
The recommendation for patients who insist on taking creatine is that they use some judgment. First, limit the daily dose to 3 grams. Many of the commercial product labels suggest much higher does. Take plenty of fluids, and if you can't take liquids for any reason, omit the creatine until you can.
A controlled trial of creatine in ALS is being planned."
Benjamin Rix Brooks, M.D.
ALS Research Center
University of Wisconsin Hospital and Clinics
Madison, WI
Michael C. Graves, M.D.
UCLA ALS Research Clinic
Reed Neurological Research Center
University of California
Los Angeles, CA
Taken from the World Federation of Neurology website
Donation of Tissue for Research - Consider giving the most precious gift
Researchers have learned a great deal about amyotrophic lateral sclerosis (ALS), a fatal neuromuscular disorder, in the past dozen years. Yet, we still do not have all the answers as to what causes ALS or how it begins. Investigators are searching for the answers to these and many other puzzling questions about ALS in their effort to find effective treatments and ways to prevent the disease.
Because ALS (often called Lou Gehrig's disease) is a disease of the motor neurons that are located in the brain and spinal cord, it is difficult to access this tissue in patients. Brain research using postmortem tissue is critical to understanding ALS. However, there can be a scarcity of ALS brain and spinal cord tissue for research studies.
Human tissue of ALS patients is the most precious resource for ALS researchers. ALS tissue when combined with complete information about the donor's ALS history will help investigators find answers. Researchers will compare ALS tissue with non-diseased tissue. They will compare tissue of patients with different histories - age of onset and time of diagnosis, progression of the disease, care and treatment during the course of the disease, and other vital information - to unravel the mystery of why and how ALS begins and then progresses to a tragic end.
Persons with ALS and their families realize the importance of research into the cause of ALS, as understanding the cause offers the best opportunity for finding and producing effective therapies to treat and/or prevent ALS in the future. Many people have already played a key role in advancing the knowledge about ALS by providing blood samples, muscle biopsies and other specimens.
For some people, this decision or commitment to donate tissue upon their death may be difficult, especially when coping with the day to day challenges of ALS. However, it may offer a sense of purpose or comfort to know that one will be contributing to the continuing quest for knowledge about ALS.
The ALS Association is currently sponsoring a program to help neurologists and select medical centers coordinate and fund tissue donations for ALS research.
To learn more about being a donor, discuss tissue donation with your primary care physician and/or your neurologist. Discuss how the donation process is carried out, particularly if you live in a city other than where your ALS clinic and neurologist are located.
For more information about where ALS tissue donation is available, contact The ALS Association at (800) 782-4747 or refer to the following list of medical centers.
A patient's lasting legacy can be the donation of brain and spinal cord tissue for ALS research.
Updated 5/03/06
California
Human Brain and Spinal Fluid Resource Center
West Los Angeles VA Medical Center
11301 Wilshire Boulevard, 127A
Los Angeles, CA 90073
W. W. Tourtellotte, MD, Director
Diane Guntrip, Coordinator
310-268-3536
310-636-5199 *
University of Southern California
Alzheimer's Disease Research Center
Department of Pathology
2011 Zonal Ave.
Los Angeles, CA 90033
Carol Miller, MD
323-442-1602
213-740-7777 *
California Pacific Medical Center
2324 Sacramento St., Ste. 150
San Francisco, CA 94115
Jason Mass, Clinical Research Nurse
415-923-3967
(At this time, tissue donation at California Pacific Medical Center is available only for patients already being seen at this ALS Center.)
ALS Specimen Bank
11645 Wilshire Blvd., Ste. 770
Los Angeles, CA 90025
310-473-5500
Elyse Singer, MD, Director
Linda DeSepulveda, PhD, RN (in UCLA ALS Ctr.)
310-825-9816
Florida
University of Miami
Brain & Spinal Cord Research Center for ALS
1550 N.W. 10th Ave., 410PAP (R-5)
Miami, FL 33136
Lillian Rodriguez, Project Coordinator
800-592-7246 *
Illinois
Northwestern University
Department of Neurology
Medical School Tarry 13-715
303 E. Chicago Ave.
Chicago, IL 60611-3008
Elena Zvirbulis
zvirbulis@northwestern.edu
312-503-0154
Kentucky
University of Kentucky
Department of Neurology
Kentucky Clinic, L445, Wing D
Lexington, KY 40536-0284
Edward J. Kasarskis, MD, PhD, Director
859-281-4920
Louisiana
LSU Health Science Center
2020 Gravier St., Ste. D
New Orleans, LA 70112-2234
Melissa Musacchia, Administrator
504-599-0916
504-584-0019 (pager)
Maryland
JHMI/Neuromuscular Division
600 N. Wolfe St.
Baltimore, MD 21287
Lora L. Clawson, MSN, CRNF
410-955-8511
(At this time, tissue donation at Johns Hopkins University is available only for patients already being seen at this ALS Center.)
Massachusetts
Day Neuromuscular Lab
MGH East, Bldg. 114
16th Street
Charlestown, MA 02129
Robert Brown, Jr., MD
617-726-5750
Minnesota
Mayo Medical Center
Mayo Foundation
Department of Neurology
200 First St., S.W.
Rochester, MN 55905
Eric J. Sorenson, MD
507-284-2511 *
507-284-8729
New York
Neurological Institute
710 W. 168th St., NI-9
New York, NY 10032
M. L. Del Bene, MS, RN, NP-P,
Clinical Administrator
212-305-1319*
Mt. Sinai Medical Center
Department of Neurology
1 Gustave L. Levy Pl.
New York, NY 10029
Melvin D. Yahr, MD
212-241-7301 *
Pennsylvania
Penn State Hershey Medical Center
Division of Neurology
500 University Drive
Hershey, PA 17033
Zachary Simmons, MD
717-531-8692
(At this time, tissue donation at Penn State Hershey Medical Center is available only for patients already being seen at this ALS Center.)
University of Pittsburgh School of Medicine
Department of Pathology - BST
S-420 3500 Terrace St.
Pittsburgh, PA 15261
Robert Bowser, PhD
412-383-7819
412-958-8312* (pager)
MCP Hahnemann
219 N. Broad St., 7th Floor
Philadelphia, PA 19107
Terry Paylor, RN
215-762-5186
215-509-9452* (pager)
The National Disease Research Interchange
1880 JFK Blvd., 11th Floor
Philadelphia, PA 19103
Lisa Morris, Sr. Mgr. Odyssey Dept.
800-222-6374 ext. 245*
They will direct the donation to anywhere in the country.
Vermont
Vermont ALS Center
C225A Given Medical Building
University of Vermont
College of Medicine
89 Beaumont Dr.
Burlington, VT 05405
Randall Beurman, Research Coordinator
802-656-4420
Also a resource for organ and tissue donation other than brain and spinal cord tissue is:
Center for Organ Recovery and Education (CORE)
204 Sigma Dr.
Pittsburgh, PA 15238
Pat Kornick, Director of Awareness and Education
800-DONORS-7 *
CORE's National office staff will direct the patient and family to a procurement center in their geographic area. Be aware that in many cases, organ and tissue donation - except for brain and spinal cord - may not be possible for people with ALS as patients must be declared "brain dead" first. Consult CORE, your local organ and tissue center or your neurologist for specific and individual information.
* Connotes a phone number that offers access 24 hours/7 days a week
Revised 7/19/02
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